Article
The unfolded protein response in models of human mutant G93A amyotrophic lateral sclerosis.
The European journal of neuroscience - 1 Mar 2012
Prell T, Lautenschläger J, Witte O W, Carri M T, Grosskreutz J
Abstract excerpt
Recent studies indicate that endoplasmic reticulum (ER) stress is involved in the pathogenesis of familial and sporadic amyotrophic lateral sclerosis (ALS). ER stress occurs when the ER-mitochondria calcium cycle (ERMCC) is disturbed and misfolded proteins accumulate in the ER. To cope with ER stress, the cell engages the unfolded protein response (UPR). While activation of the UPR has been shown in some ALS...
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